A person with a hereditary deficiency realated to mitochondrial fatty acid oxidation shows accumulation of a long-chain acylcarnitine in the mi- tochondrial matrix. What factor could be responsible for this disorder? Provide a biochemical explanation for its mechanism of action.
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- The interconverison of DHAP and GAP greatly favors the formation of DHAP at equilibirum. Yet the conversion of DHAP by triose phosphate isomerase proceeds readily. Why?The mitochondrial Pyruvate Dehydrogenase Complex (PDC) is a very large enzyme complex which catalyses the oxidative decarboxylation of pyruvate to acetyl-CoA. Describe the enzyme components that make up the PDC, indicating the contribution each makes to the pyruvate to acetyl-CoA process, as well as how the PDC itself is regulated by intermediates?What structurtal property of mono- and polyunsaturated fatty acids prevents oxidation of these compounds by the β-oxidation pathway? what additional types of reactions are needed?
- Describe the effects of (1) oligomycin and (2) uncouplers of oxidative phosphorylation, e.g., dinitrophenol (DNP), carbonyl cyanide-p- trifluoromethoxyphenylhydrazone (FCCP), on respiration and ATP synthesis when added to a suspension of mitochondria with excess malate, ADP, and inorganic phosphate (Pi).Considering that triacylglycerols get hydrolyzed in the small intestine because of pancreatic lipases, write the reactions using structural formulas by which a 7-carbon fatty acid is activated and oxidized in the liver of this patient. Indicate cofactors and underscore the final CoA products.In fatty acid biosynthesis explain the following two parts -(a) What effect will an increase in oxaloacetate in mitochondria have on fatty acid biosynthesis?(b) How insulin and glucagon affect fatty acid oxidation and biosynthesis reactions/pathways, name them.
- Is it true that beta-ketoacyl-ACP-reductase catalyzes the committed stepn in fatty acid biosynthesis?Name the molecules used for gluconeogenesis. What are the sources of these molecules? Under what conditions would gluconeogenesis occur?Dicyclohexylcarbodiimide (DCCD) is a reagent that reacts with Asp or Glu residues. Explain why the reaction of DCCD with the c subunits of F1F0-ATP ase blocks its ATP-synthesizing activity.
- Is the statement, "citrate carries acetyl CoA to the mitochondrial matrix which is the site of fatty acid biosynthesis , incorrect?Explain why people with a hereditary deficiency of carnitine acyltransferase II have muscle weakness. Why are the symptoms more severe during fasting?Looking at the structure of alpha linolenic acid, how many rounds of beta oxidation will occur to fully oxidize the fatty acid into acetyl CoA molecules? Please provide explanations